Infantile form of meningeal angiomatosis with sudanophilic leucodystrophy associated with complex abiotrophies Study of a second family

Abstract The authors describe the clinical and pathological features of 2 familial cases of a syndrome previously described by Hooft et al (1965). Clinically, the onset of the disease is during the first year of life, the course is rapid and the main features are mental deterioration and convulsive seizures resistant to all forms of therapy. Pathologically, the condition is characterized by: (1) leptomeningeal capillary-venous angiomatosis; (2) sudanophilic leucodystrophy; (3) a systemic abiotrophic process involving mainly areas of thalamus, cerebellum and brain-stem.